Background: The dizygotic monochorionic twins complicated with twin-twin transfusion syndrome were thought as rare events. Objective: To report a case of twin-twin transfusion syndrome in a dizygotic monochorionic diamniotic twin that was successfully treated using fetoscopic laser photocoagulation, as well as to review the published literature on dizygotic monochorionic twins with twin-twin transfusion syndrome. Search Strategy: Combinations of “dizygotic monochorionic” “twin-twin transfusion syndrome” were used as keywords with search engine PubMed. Articles were reviewed for year of publication and extraction and analysis of the same data. Selection Criteria: Cases of twin-twin transfusion syndrome in a dizygotic monochorionic diamniotic twin gestation treated using fetoscopic laser photocoagulation were included. Data Collection and Analysis: Basic characteristics and outcome of dizygotic monochorionic diamniotic twins were collected and analyzed. Main results: The pregnancy woman delivered vaginally at 32+3 weeks of gestation due to premature rupture of membrane. Both babies had normal-appearing external genitalia. The literature identified 39 dizygotic monochorionic twins and 8 (20%) of the 40 cases (including the current case) complicated by twin-twin transfusion syndrome. Four cases undergoing laser therapy and three cases resulted in live births. Conclusions: The possibility of dizygotic twins should be considered when encountering monochorionic twin pregnancies with different fetal sex. Fetoscopic laser surgery could be used in dizygotic monochorionic twin pregnancy with twin-twin transfusion syndrome.